Measuring Cognitive and Behavioural Change in ALS

Study titleCognitive and behavioural impairment in Amyotrophic Lateral Sclerosis: Comparing measures and validating revised consensus diagnostic criteria for Amyotrophic Lateral Sclerosis-Frontotemporal Spectrum Disorder
UK Chief InvestigatorProfessor Laura Goldstein
Research summaryAbout 50% of people with Amyotrophic Lateral Sclerosis (ALS) (also known as Motor Neuron Disease (MND)) develop changes in their thinking and behaviour which may, in around 15% of cases, lead to the diagnosis of frontotemporal dementia (FTD). Several different assessment methods - called screening tools – exist for testing people with ALS and obtaining information from carers in clinic/at home. However, it is unclear whether each of the different tools would lead to the same conclusion in the same person with ALS, since they use different approaches and have different rules for deciding whether someone is impaired. If they do not agree, some people could receive an incorrect diagnosis of changes in their thinking, language and behaviour, depending on which tool was used; this might affect the practical care they receive. Thus, the main aim of this project is to test whether different screening tools lead to similar rates of diagnosed change in the same people. We will test this by giving three screening tests measuring thinking and language to about 114 people with ALS. About 91 people who know the person with ALS well will complete sets of questions about any change in the behaviour of the person with ALS. We can then examine differences in how these measures detect thinking and behaviour change. The people with ALS and about 100 healthy controls (similar to the people with ALS in age, gender and education) will also complete other measures of thinking and language so we can i) compare how each screening measure compares to a common set of measures of thinking and language, ii) determine which is the best screening test of thinking and behaviour changes in people with ALS and iii) inform clinicians and researchers about the implications of using different measures in their clinical work and research.
Inclusion/exclusion criteriaINCLUSION CRITERIA
People with ALS:
age at least 18 years; El-Escorial-defined ALS; ability to give informed consent; availability, wherever possible, of knowledgeable informant to complete the behavioural assessment.

Healthy controls:
Age-, gender- and years of education-matched healthy controls from local community sources with other similar non-ALS-related inclusion and exclusion criteria.

Age at least 18 years old; ability to give informed consent.

Diagnosis of Progressive Muscular Atrophy or Primary Lateral Sclerosis.

For people with ALS and controls:
Age greater than 75 years as this makes it more likely that other disorders may impair test performance; history of head injury or stroke or other neurological/medical/psychiatric disease likely to impair cognition; respiratory impairment sufficient to impair cognition and uncorrected by Noninvasive ventilation; not having English as first language; receiving treatment for other life-limiting illness (e.g. cancer); inability to give informed consent.

Inability to complete the questionnaires independently.
Current statusClosed to recruitment
LocationsLondon (King's College Hospital)
London (The Royal London Hospital)
Royal Sussex County Hospital & Princess Royal Hospital, Brighton
London (St George's Hospital, Tooting)
Contact detailsLyndsay Didcote, Email:
Recruitment group(s)Participant groups:
1. Patients with MND
2. Neurologically healthy controls
3. Carers of people with MND.
Recruitment target(s)114 patients with MND, 100 controls, 91 carers.
Key datesActual opening date: 28 January 2019
Recruitment planned end date: 31 March 2021.
Actual closure date: 30 June 2021
Funder(s)Motor Neurone Disease Association (MNDA)
SponsorKing's College London
Study designObservational (validation of outcome measures)
Intervention (if applicable)Not applicable
Phase (if applicable)Not applicable
Outcome measuresThe primary objective is to compare the ability of ALS-specific screening assessments to identify people with Amyotrophic Lateral Sclerosis as cognitively or behaviourally impaired or having ALS-Frontotemporal dementia (ALS-FTD) to answer the questions:

1) Which is the best neuropsychological screening test of cognitive impairment in ALS?
2) How do behavioural screens compare in identifying impairment? and
3) What are the implications for the new consensus criteria for diagnosing ALS frontotemporal syndromes and for research and for clinical care?
Publications / Results reportsLinks will be provided when papers are published.
Participant Information Sheet